Pediatric Autoimmune Hepatitis

Purpose of Review: Autoimmune hepatitis (AIH) is a chronic progressive autoinflammatory disease of the liver caused by the dysregulation of an individual’s immune response. As discovery in science and innovation in clinical care have been reported, we look to summarize the most up-to-date clinical advances and management strategies in pediatric AIH. Recent Findings: Discovery related to genetic predisposition, epigenetic modification, and environmental triggers has been implicated in the etiopathogenesis of AIH and enabled a greater understanding of the disease and its management. Still, diagnosis in children is dependent on adult-derived scoring systems that are invasive and imperfect. Treatment regimens with immunosuppressive agents have been well established and initial responses are generally favorable. However, side effects can be significant, medication titration can be variable, and relapses are common. Medication intolerant or refractory cases present unique challenges in both prognosis and alternative therapeutic opportunities. Summary: In children, improvements in strategies that encompass diagnostic and prognostic tools to optimize care and predict outcomes, as well as establishing learning health network approaches to care constitute areas of ongoing and future discovery. © 2023, The Author(s), under exclusive licence to Springer Nature Switzerland AG.